Case Report: Effect of DBS in a Patient With Cerebellar Atrophy

ObjectiveThe aim of this study is to present a case report of deep brain stimulation (DBS) in the globus pallidus internus (GPi) and ventral anterior nucleus of the thalamus (VA) in a patient with dystonic tremor due to progressive cerebellar atrophy of unknown etiology.BackgroundAlthough DBS has been shown to be effective in disorders of basal ganglia origin, its use for cerebellar origin disorders remains less understood. This case study presents a patient with dystonic tremor, ataxia, and psychiatric symptoms associated with cerebellar atrophy of unknown etiology. The patient was treated with DBS implantation in GPi and VA. Targets were selected using clinical observation and data obtained from stereoelectroencephalography (sEEG) depth electrode implantation and subsequent observation in an inpatient Neuromodulation Monitoring Unit (NMU).MethodsPreoperative and 1-year postoperative scores were examined to determine improvement in symptoms using Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS), Barry Albright Dystonia Scale (BADS), and Scale for Assessment and Rating of Ataxia (SARA).ResultsAn improvement in movement symptomatology was seen 1 year after permanent electrode implantation and programming using the movement scales (BFMDRS movement preprocedure 15.5, postprocedure 8; BFMDRS disability preprocedure 13, postprocedure 8; BADS preprocedure 14, postprocedure 7; SARA preprocedure 20.5, postprocedure 14.5).ConclusionsDBS can have beneficial effects on multiple symptoms associated with cerebellar degeneration. We cannot exclude the possibility of other neurologic deficits in this patient, and thus the effect of DBS could be mediated through multiple mechanisms. This case report suggests the need for further exploration of the role of DBS in cerebellar-origin disorders.